title: Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort.
abstract: BACKGROUND
  Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.
  
  
  METHODS
  From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X-rays.
  
  
  RESULTS
  Thirty-seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow-up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI-related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi-system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5-year survival was 87.1%, and immunodeficiency was not detected as a sequel.
  
  
  CONCLUSIONS
  MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications.
authors.affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.
authors.forename: Stephane
authors.initials: S
authors.lastname: Ducassou
authors.forename: Fanny
authors.initials: F
authors.lastname: Seyrig
authors.forename: Caroline
authors.initials: C
authors.lastname: Thomas
authors.forename: Anne
authors.initials: A
authors.lastname: Lambilliotte
authors.forename: Perrine
authors.initials: P
authors.lastname: Marec-Berard
authors.forename: Claire
authors.initials: C
authors.lastname: Berger
authors.forename: Genevieve
authors.initials: G
authors.lastname: Plat
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugiere
authors.forename: Marie
authors.initials: M
authors.lastname: Ouache
authors.forename: Mohamed
authors.initials: M
authors.lastname: Barkaoui
authors.forename: Corinne
authors.initials: C
authors.lastname: Armari-Alla
authors.forename: Patrick
authors.initials: P
authors.lastname: Lutz
authors.forename: Guy
authors.initials: G
authors.lastname: Leverger
authors.forename: Xavier
authors.initials: X
authors.lastname: Rialland
authors.forename: Ludovic
authors.initials: L
authors.lastname: Mansuy
authors.forename: Helene
authors.initials: H
authors.lastname: Pacquement
authors.forename: Eric
authors.initials: E
authors.lastname: Jeziorski
authors.forename: Virginie
authors.initials: V
authors.lastname: Gandemer
authors.forename: François
authors.initials: F
authors.lastname: Chalard
authors.forename: Jean François
authors.initials: JF
authors.lastname: Chateil
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.forename: Jean François
authors.initials: JF
authors.lastname: Emile
authors.forename: Jean
authors.initials: J
authors.lastname: Donadieu
mesh_terms: D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008482:Mediastinum / Q000473:pathology; D013950:Thymus Gland / Q000473:pathology*
keywords: Langerhans cell histiocytosis; mediastinal nodes; survey; thymus